SJORGEN’S SYNDROME

-Definition/introduction

-Clinical features

-Histopathology

-Diagnosis

-Treatment

Introduction

 Sjorgen’s syndrome is a chronic, systemic auto-immune disorder that principally involves the
salivary and lacrimal gland resulting in xerostomia (dry mouth) and xeropthalmia (dry eye).

-It is caused by the lymphocytic mediated destruction of lacrimal and salivary gland parenchyma.

-The effect on the eye is called keratoconjunctivitis sicca.

-The clinical presentation of both the xerostomia and xeropthalmia is called sicca syndrome.

It can be further classified into;

 Primary Sjorgen’s syndrome; Sicca syndrome alone with no other autoimmune disorders
present.
 Secondary Sjorgen’s syndrome; Sicca syndrome+ other associated autoimmune diseases
(Systemic Lupus Erythematosus, rheumatoid arthritis etc).

Epidemiology

-Affects middle age mostly

-90% occur in women

-Peak age;50yrs

Aetiology

 No known aetiology but certain HLAs have been implicated such as HLA-DRw52, also viruses like
Epstein-barr, retrovirus have also been implicated but not clinically proven

Clinical features
 Xerostomia (dry mouth); this can account for other features like

a) Dysgeusia

b) Fissured tongue

c) Difficulty swallowing

d) Difficulty with wearing denture

e) Candidiasis (oral thrush)

f) Dental caries

g) Infections

 Bilateral enlargement of major salivary gland in some, can be intermittent/persistent in nature.

 Defect in the ocular surface epithelium which can result in blurred vison, aching pain. ( NB; these
ocular manifestations are less severe in the morning on wakening but become more
pronounced as the day progress.

Histopathology

- A benign lymphocytic infiltrate replaces major salivary gland parenchyma

- Initial lesion is focal periductal aggregation of lymphocytes and occasionally plasma cells
- As inflammatory foci enlarge, a corresponding level of acinar degeneration is seen with increase
lymphocytic infiltration
- Acini is destroyed but ductal epithelium persists
- Presence of epimyoepithelial islands
Benign Lymphoepithelial Lesion in Sjögren Syndrome.

Sjögren Syndrome. Labial gland biopsy showing multiple

lymphocytic foci.

Diagnosis

It depends on the correlation between patient history, laboratory data and clinical examination

-laboratory + clinical

-biopsy of the minor salivary glands of the lower lips.

Treatment

 Management of the symptoms

-Artificial saliva and artificial tears are available for this purpose.

-Preventive oral measures are extremely important relative to xerostomia.

- Scrupulous oral hygiene, dietary modification, topical fluoride therapy, and remineralizing solutions are
important in maintaining oral and dental tissues.

-Use of sialagogues, such as pilocarpine and cevimeline, remains of limited value, especially in long-
standing Sjogren's syndrome.